心肌淀粉样变性 myocardial amyloidosis - PubMed 文献(第 5 页)
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心肌淀粉样变性 的 PubMed 搜索结果(第 5 页)
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41. Extra-articular manifestations of rheumatoid arthritis: An update.
41. 类风湿性关节炎的关节外表现：最新进展PMID:日期:2011-09-10[中文摘要] 类风湿关节炎（RA）是一种免疫介导的疾病，涉及慢性低度炎症，可能逐渐导致关节破坏、畸形、残疾甚至死亡。尽管其主要表现为骨关节及关节周围病变，但RA是一种全身性疾病，常伴有皮肤及器官特异性关节外表现（EAM）。尽管已在众多RA队列中对EAM进行了研究，但其定义或分类尚无统一标准。本文就EAM的发病率、临床特点及当前治疗方法等方面的现有认识进行综述。在初步分类尝试中，我们将EAM与RA合并症及系统性炎症的一般性、全身性表现加以区分。此外，我们将EAM分为皮肤型和内脏型，二者又各分为重度和非重度。在来自12个大型RA队列的汇总数据中，伴有EAM的患者，尤其是重度EAM患者，其合并症发生率和死亡率均高于无EAM的患者。理解EAM的复杂性及其管理对临床医生而言仍是一项挑战，尤其是因为药物治疗对EAM的疗效尚未在随机临床试验中得到系统评估。
[英文摘要] Rheumatoid arthritis (RA) is an immune-mediated disease involving chronic low-grade inflammation that may progressively lead to joint destruction, deformity, disability and even death. Despite its predominant osteoarticular and periarticular manifestations, RA is a systemic disease often associated with cutaneous and organ-specific extra-articular manifestations (EAM). Despite the fact that EAM have been studied in numerous RA cohorts, there is no uniformity in their definition or classification. This paper reviews current knowledge about EAM in terms of frequency, clinical aspects and current therapeutic approaches. In an initial attempt at a classification, we separated EAM from RA co-morbidities and from general, constitutional manifestations of systemic inflammation. Moreover, we distinguished EAM into cutaneous and visceral forms, both severe and not severe. In aggregated data from 12 large RA cohorts, patients with EAM, especially the severe forms, were found to have greater co-morbidity and mortality than patients without EAM. Understanding the complexity of EAM and their management remains a challenge for clinicians, especially since the effectiveness of drug therapy on EAM has not been systematically evaluated in randomized clinical trials.
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42. Echocardiographic and electrocardiographic presentations of patients with endomyocardial biopsy-proven cardiac amyloidosis.
42. 经心内膜心肌活检证实的心脏淀粉样变性患者的超声心动图及心电图表现PMID:期刊:The Medical journal of Malaysia PubMed日期:2018-12[中文摘要] 心脏淀粉样变性诊断不足，其患病率尚不明确。这是一项回顾性、非随机、单中心研究，研究对象为经心内膜心肌活检证实的心脏淀粉样变性患者，重点关注其超声心动图和心电图（ECG）表现。这是马来西亚关于该主题的首个病例系列。我们纳入了2010年1月至2018年1月期间所有经心内膜心肌活检证实的心脏淀粉样变性患者，并回顾了其病历资料。所有患者的超声心动图和心电图检查结果均经过回顾分析，并与基础人群均值进行比较。共有13例经活检证实的心脏淀粉样变性患者。所有活检均显示轻链（AL）淀粉样蛋白。多数患者（8例，61.5%）为男性，大多数患者（8例，61.5%）为华裔。我们对其进行了变形成像检查的全部7例患者，纵向应变超声心动图均显示心尖保留模式。平均射血分数为49.3%（SD=7.9）。所有患者均有向心性左心室肥厚和右心室肥厚。所有患者均存在舒张功能障碍，其中13例中有9例（69.2%）具有限制性充盈模式（E/A ≥2.0，E/e' ≥15）。心电图上，12例（92%）患者PR间期延长（中位数200ms，IQR 76.50ms），9例（69.2%）患者有假性梗死模式。超声心动图在诊断心脏淀粉样变性中发挥着重要作用。在负荷条件未增加的情况下，出现向心性左心室肥厚伴射血分数保留，应提醒临床医生考虑该病的可能性。右心室肥厚，尤其是纵向应变成像显示心尖保留模式，可进一步支持这一判断。
[英文摘要] Cardiac amyloidosis is under diagnosed and its prevalence is unknown. This is a retrospective, nonrandomised, single centre study of patients with endomyocardial biopsy-proven cardiac amyloidosis focusing on their echocardiographic and electrocardiogram (ECG) presentations. This is the first case series in Malaysia on this subject. We identified all of our endomyocardial biopsyproven cardiac amyloidosis patients from January 2010 to January 2018 and reviewed their medical records. All patients echocardiographic and ECG findings reviewed and analysed comparing to basic mean population value. In total there are 13 biopsy-proven cardiac amyloidosis patients. All of the biopsies shows light chain (AL) amyloid. Majority of the patients (8, 61.5%) is male, and most of our patients (8, 61.5%) is Chinese. All seven patients on whom we performed deformation imaging have apical sparing pattern on longitudinal strain echocardiogram. Mean ejection fraction is 49.3%, (SD=7.9). All patients have concentric left ventricular hypertrophy and right ventricular hypertrophy. Diastolic dysfunction was present in all of our patients with nine out of 13 patients (69.2%) having restrictive filling patterns (E/A ≥2.0 E/e' ≥15). On electrocardiogram, 12 (92%) patients have prolonged PR interval (median 200ms, IQR 76.50ms) and 9 (69.2%) patients have pseudoinfarct pattern. Echocardiography plays an important role in diagnosing cardiac amyloidosis. The findings of concentric left ventricular hypertrophy with preserved ejection fraction without increased in loading condition should alert the clinician towards its possibility. This is further supported by right ventricular hypertrophy and particularly longitudinal strain imaging showing apical sparing pattern.
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43. Infiltrative cardiomyopathy with conduction disease and ventricular arrhythmia: electrophysiologic and pathologic correlations.
43. 伴传导疾病和室性心律失常的浸润性心肌病：电生理与病理学相关性PMID:日期:1980-01[中文摘要] 本文描述了两例病例，一例为心脏结节病，另一例为原发性心脏淀粉样变性，并将电生理研究与尸检传导系统研究进行了相关性分析。病例1中，心电图显示右束支传导阻滞，伴一度及间歇性三度房室（A-V）传导阻滞以及反复发作的单灶性阵发性室性心动过速。电生理检查发现窦性心律正常，但A-H间期（175 ms）和H-V间期（60 ms）延长，且额外刺激可诱发重复性心室激动。尸检显示左心室后壁结节病性动脉瘤，以及房室结、希氏束和束支的肉芽肿性浸润。病例2中，心电图显示窦性心动过缓、S-T间期延长及反复发作的室性颤动。电生理检查显示窦房结恢复时间延长（6,080 ms）及H-V间期延长（85 ms）。尸检显示窦房结、心房、近端束支及左右心室心肌有明显淀粉样物质浸润。因此，电生理检查与病理学发现之间存在极好的相关性。这些病例表明，对该疾病作出临床诊断具有重要意义，因为在某些病例中，除抗心律失常治疗外，还可能允许进行特定的治疗干预。
[英文摘要] Two cases are described, one of cardiac sarcoidosis and another of primary cardiac amyloidosis, in which correlation was made between electrophysiologic and postmortem conduction system studies. In Case 1 the electrocardiogram revealed right bundle branch block with first degree and intermittent third degree atrioventricular (A-V) block and recurrent unifocal paroxysmal ventricular tachycardia. Electrophysiologic studies disclosed normal sinus rhythm with prolonged A-H (175 ms) and H-V (60 MS) intervals and extrastimulus induction of repetitive ventricular firing. Postmortem examination revealed a sarcoid aneurysm of the posterior left ventricle and granulomatous infiltration of the A-V node, His bundle and bundle branches. In case 2 the electrocardiogram revealed sinus bradycardia, a prolonged S-T interval and recurrent ventricular fibrillation. Electrophysiologic studies demonstrated a prolonged sinus nodal recovery time (6,080 ms) and H-V (85 ms) interval. Postmortem examination revealed marked amyloid infiltration of the sinoatrial node, atria, proximal bundle branches and left and right ventricular myocardium. There was thus excellent correlation between electrophysiologic and pathologic findings. These cases indicate the importance of making a clinical diagnosis of the disease that might in some cases allow specific therapeutic intervention in addition to antiarrhythmic therapy.
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44. Treatment of primary systemic amyloidosis (AL): role of intensive and standard therapy.
44. 原发性系统性淀粉样变性（AL）的治疗：强化治疗和标准治疗的作用PMID:日期:2012-10[中文摘要] 免疫球蛋白轻链（AL）淀粉样变性是一种克隆性浆细胞疾病。诊断延迟是改善AL患者预后的主要障碍。几乎所有系统性AL患者都需要细胞毒性治疗。治疗可以改善症状和生活质量，并延长生存期。支持治疗是治疗方案中不可或缺的组成部分。心脏受累的严重程度是判断预后的重要决定因素，并影响治疗选择。心脏生物标志物和血清游离轻链检测是评估预后和监测治疗反应的重要工具。大剂量美法仑化疗联合自体干细胞解救似乎能带来生存获益；然而，这仅适用于四分之一的AL患者。标准剂量的类固醇和烷化剂联合化疗是一种安全有效的治疗策略，可使许多患者的器官功能得到改善。硼替佐米和来那度胺等新型药物已显示出有前景的活性，目前正在临床试验中作为联合方案的一部分进行评估。
[英文摘要] Immunoglobulin light-chain (AL) amyloidosis is a clonal plasma cell dyscrasia. Delay in diagnosis is the major hurdle in improving the outcomes of AL patients. Almost all patients with systemic AL need cytotoxic therapy. Treatment can improve symptoms and quality of life, as well as extend survival. Supportive care is an integral part of the treatment plan. Severity of cardiac involvement is an important determinant of prognosis and influences the choice of therapy. Cardiac biomarkers and serum free light chain assay are important tools for assessing prognosis and monitoring treatment response. Myeloablative chemotherapy with melphalan and autologous stem cell rescue appears to offer survival benefit; however, it is an option for only a quarter of AL patients. Standard-dose combination chemotherapy with steroids and alkylating agents is a safe and effective treatment strategy that can result in improvement of organ function in many patients. Newer agents such as bortezomib and lenalidomide have shown promising activity and are being evaluated as part of combination regimens in clinical trials.
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45. Fundamentals of Light Chain Cardiac Amyloidosis: A Focused Review.
45. 轻链心脏淀粉样变基础：重点综述PMID:期刊:Cardiovascular & hematological disorders drug targets PubMed日期:2020[中文摘要] 在美国，AL型心脏淀粉样变性（AL CA）的估计患病率约为每百万8至12例。在美国，近30%至50%确诊的AL型淀粉样变性病例存在多系统受累，包括心脏受累。即使有先进的诊断检测和新疗法可用，预后仍然较差。它作为射血分数保留型心力衰竭的病因常被忽视，导致诊断延迟，而此时治疗选择有限且与不良生存结局相关。因此，需要对医生进行教育，以确保其被高度重视并纳入鉴别诊断。本文的目的是综述心脏淀粉样变性在诊断和管理方面的进展，旨在教育在初级保健环境中提供医疗服务的同行。我们总结了淀粉样变性的发病机制、其与浆细胞病的关联、新型诊断和监测方法（包括超声心动图、心血管磁共振成像、组织病理学技术、系统性生物标志物），以及先进的治疗方法（包括支持性对症治疗和针对淀粉样蛋白沉积的标准治疗化疗）。鉴于淀粉样变性总体预后较差，我们还讨论了姑息治疗和临终关怀的作用。
[英文摘要] The estimated prevalence of AL CA in the US is approximately 8-12 cases per million. Almost 30-50% diagnosed cases of AL amyloid in the US have multisystem involvement, including cardiac involvement. Even with the availability of advanced diagnostic testing and novel therapies, prognosis remains poor. It is overlooked as a cause of heart failure with preserved ejection fraction leading to a delay in diagnosis when management options are limited and associated with poor survival outcomes. Therefore, the education of physicians is needed to ensure that it would be highly considered as a differential diagnosis. The purpose of this manuscript is to review the advances in the diagnosis and management of cardiac amyloidosis with the aim of educating colleagues who provide care in the primary care setting. We have summarized the pathogenesis of amyloidosis, its association with plasma cell dyscrasias, novel diagnostic and surveillance approaches including echocardiography, cardiovascular magnetic resonance imaging, histopathologic techniques, systemic biomarkers, and advanced treatment approaches including supportive symptomatic management and standard of care chemotherapy targeting the amyloid deposits. Given the overall poor prognosis of amyloidosis, we have also discussed the role of palliative and hospice care.
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46. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis.
46. 心脏淀粉样变性的M型和二维超声心动图特征PMID:日期:1981-01[中文摘要] 对28例心脏淀粉样变性患者进行了超声心动图检查——26例采用M型超声，13例采用二维（2D）超声研究。所有患者均有心力衰竭，且经活检证实为淀粉样变性。M型超声特征包括：（1）所有患者左心室（LV）内径正常；（2）室间隔增厚（88%）、左心室后壁增厚（77%）、右心室（RV）前壁增厚（79%）；（3）室间隔增厚率降低（96%）、左心室后壁增厚率降低（65%）及左心室整体功能减低（62%）；（4）左心房扩大（50%）；（5）心包积液（58%）。二维超声心动图提供了额外特征：（1）乳头肌增厚（13例中5例）；（2）瓣膜增厚（13例中4例）；（3）对增厚的右心室壁显示更佳；（4）增厚的心壁呈特征性的“颗粒状闪烁”表现——推测继发于淀粉样物质沉积——13例中12例可见此征象。因此，M型超声心动图有助于识别心脏淀粉样变性。然而，对于不明原因心力衰竭的患者，二维超声心动图能更好地显示增厚的心壁并伴有“颗粒状闪烁”表现，这几乎可确诊为心脏淀粉样变性。
[英文摘要] Twenty-eight patients with cardiac amyloidosis were studied by echocardiography -- 26 by M-mode and 13 by two-dimensional (2D) studies. All had heart failure and biopsy-proved amyloidosis, M-mode features included (1) normal left ventricular (LV) dimension in all; (2) thickened ventricular septum (88%), LV posterior wall (77%), and right ventricular (RV) anterior wall (79%); (3) decreased thickening of ventricular septum (96%) and of LV posterior wall (65%) and reduced LV global function (62%); (4) left atrial enlargement (50%); and (5) pericardial effusion (58%). Two-dimensional echocardiography provided additional features: (1) thickened papillary muscles (five of 13); (2) thickened valves (four of 13); (3) better appreciation of thickened RV wall; and (4) a characteristic "granular sparkling" appearance of thickened cardiac walls -- presumably secondary to the amyloid deposit -- which was noted in 12 of 13 patients. Thus, M-mode echocardiography is helpful in the recognition of cardiac amyloidosis. However, the better appreciation with 2D echocardiography of thickened cardiac walls with a "granular sparkling" appearance in patients with unexplained cardiac failure is virtually diagnostic of cardiac amyloidosis.
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47. Cardiac Amyloidosis-Challenging Diagnosis and Unclear Clinical Picture.
47. 心脏淀粉样变性——具有挑战性的诊断和不明朗的临床图景PMID:日期:2021-05-06[中文摘要] 心脏淀粉样变性（CA）是一种罕见的系统性疾病，由淀粉样蛋白在心脏的细胞外沉积所致。该蛋白可积聚于心脏的任何部位：心肌、血管、心内膜、瓣膜、心外膜和壁层心包。CA的类型包括以下几种：轻链型（AL）、淀粉样蛋白A型（AA）和转甲状腺素蛋白型（ATTR）。检测特定亚型对于实施靶向治疗仍具有极其重要的意义。我们报告一例65岁女性患者，因运动能力严重下降、双侧生理性肺泡呼吸音减弱、腹水及双下肢水肿入院。因超声心动图检查结果怀疑CA，进而行进一步检查并最终确诊。本研究旨在提高临床医生对该病的认识，缩短从首发症状到确诊之间的延迟，从而改善预后。
[英文摘要] Cardiac amyloidosis (CA) is a rare systemic disease determined by the extracellular deposition of amyloid protein in the heart. The protein can accumulate in any part of the heart: myocardium, vessels, endocardium, valves, epicardium and parietal pericardium. The types of CA include the following types: light chain (AL), amyloidosis AA (Amyloid A) and transthyretin (ATTR). The detection of specific subtypes remains of great importance to implement the targeted treatment. We present the case of a 65-year-old woman, who was admitted with severe deterioration of exercise capacity, a bilateral reduction of physiological vesicular murmur, ascites and edema of lower extremities. CA was suspected due to echocardiographic examination results, which led to further examination and final diagnosis. The aim of this study is to improve the disease awareness among clinicians and shorten the delay between the first symptoms and the diagnosis establishment resulting in a better outcome.
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48. Right ventricular involvement in transthyretin amyloidosis.
48. 转甲状腺素蛋白淀粉样变性中的右心室受累PMID:期刊:日期:2018-09-07[中文摘要] 转甲状腺素蛋白淀粉样变性（ATTR）中右心室（RV）受累的程度尚不明确。本研究旨在确定ATTR淀粉样变性中右心室受累的程度，并将结果与肥厚型心肌病（HCM）中的右心室受累情况进行比较。本研究纳入了42例经超声心动图证实存在心脏淀粉样变的ATTR淀粉样变性患者（心脏ATTR）、19例左心室（LV）壁厚度正常的ATTR患者（非心脏ATTR）、25例确诊HCM的患者以及30例健康对照。记录常规参数的超声心动图测量值，以及右心室整体和节段应变。在比较心脏ATTR淀粉样变性与HCM患者的右心室结构和功能时，仅节段应变在两组之间存在差异。在心脏ATTR淀粉样变性中，我们发现右心室存在从心尖到基底部的应变梯度，其形变在心尖部最高。该模式在HCM患者中则相反。右心室受累在心脏ATTR患者中很常见。本研究还检测到ATTR心肌病患者存在右心室心尖保留模式，与此前在这些患者左心室中描述的模式相似。该模式在HCM患者中未见。需要进一步研究以评估这些发现的临床意义。
[英文摘要] The extent of right ventricular (RV) involvement in transthyretin amyloidosis (ATTR) is unknown. This study sought to establish the degree of RV involvement in ATTR amyloidosis, and compare findings with RV involvement in hypertrophic cardiomyopathy (HCM). Forty-two patients with ATTR amyloidosis and echocardiographic evidence of cardiac amyloidosis (cardiac ATTR), 19 ATTR patients with normal left ventricular (LV) wall thickness (non-cardiac ATTR), 25 patients with diagnosed HCM and 30 healthy controls were included in this study. Echocardiographic measurements for conventional parameters, as well as RV global and segmental strain, were recorded. When comparing RV structure and function between cardiac ATTR amyloidosis and HCM patients, only segmental strain differed between the two groups. In cardiac ATTR amyloidosis, we found an RV apex-to-base strain gradient with highest deformation in the apex. This pattern was reversed in patients with HCM. RV involvement is common in cardiac ATTR patients. The present study also detected an RV apical sparing pattern in patients with ATTR cardiomyopathy, similar to what has previously been described for the left ventricle in these patients. This pattern was not seen in HCM patients. Further studies are needed to assess the clinical importance of these findings.
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49. Neutrophil proteases associated with amyloid fibrils.
49. 与淀粉样纤维相关的中性粒细胞蛋白酶PMID:日期:1993-11-30[中文摘要] 我们发现，从五种不同类型系统性淀粉样变性患者中分离出的淀粉样原纤维，伴有大量具有人中性粒细胞蛋白酶——弹性蛋白酶（elastase）和组织蛋白酶G（cathepsin G）——特征的酶活性。来自淀粉样A蛋白、淀粉样转甲状腺素蛋白和淀粉样β2-微球蛋白淀粉样变性患者组织切片中的淀粉样沉积物，用抗弹性蛋白酶和组织蛋白酶G抗血清染色也呈阳性。弹性蛋白酶和组织蛋白酶G存在于中性粒细胞的嗜天青颗粒中，也较少地存在于单核细胞中，它们可能在淀粉样原纤维形成之前或形成过程中与淀粉样前体蛋白结合。这可能发生在一个细胞外炎症微环境中或吞噬溶酶体内，并在原纤维的形成中发挥作用。
[英文摘要] We found significant amounts of enzymatic activity characteristic of the human neutrophil proteases, elastase and cathepsin G, associated with isolated amyloid fibrils from patients with five different types of systemic amyloidosis. Amyloid deposits in tissue sections from the patients with amyloid A, amyloid transthyretin and amyloid beta 2-microglobulin amyloidosis also stained positive with antiserum to elastase and cathepsin G. Elastase and cathepsin G, found in the azurophilic granules of the neutrophil and, to a lesser extent, the monocyte, may become associated with amyloid precursor proteins before or during the formation of amyloid fibrils. This may occur in an extracellular inflammatory microenvironment or in a phagolysosome and play a role in the formation of the fibrils.
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50. Wild-type ATTR amyloidosis may be associated with unexpected death among the elderly.
50. 野生型ATTR淀粉样变性可能与老年人中的意外死亡相关PMID:期刊:Legal medicine (Tokyo, Japan) PubMed日期:2019-10-31[中文摘要] 野生型ATTR淀粉样变性（ATTR-wt）的特征是淀粉样物质在心脏中沉积，导致致死性心力衰竭和心律失常。本研究对556例60岁以上法医尸检病例的心脏淀粉样物质沉积进行了调查。ATTR-wt的患病率为5.8%（556例中32例），且患病率随年龄增长而升高。我们发现80岁以上人群中ATTR-wt特异性患病率为12.3%，而90岁以上人群的患病率为34.9%。这32例病例中无一例在生前作出ATTR-wt的临床诊断。在32例ATTR-wt阳性病例中，29例存在明显的外因性死亡原因，包括烧伤、溺水、低体温、自杀和交通事故。另一方面，32例中有3例确认ATTR-wt所致心力衰竭为死亡原因。这提示ATTR-wt可能与老年人的意外死亡有关。
[英文摘要] Wild-type ATTR amyloidosis (ATTR-wt) is characterized by the accumulation of amyloid in the heart, leading to fatal heart failure and arrhythmia. In this study, we investigated the amyloid deposits in the heart from 556 forensic autopsy cases over 60 years of age. The prevalence of ATTR-wt was 5.8% (32 of the 556), with the prevalence increasing as a function of age. We identified an ATTR-wt-specific morbidity rate of 12.3% for patients over 80 years of age, while the prevalence among individuals over 90 years of age was 34.9%. In none of these 32 cases had a clinical diagnosis of ATTR-wt been made. In 29 of the 32 cases found to be ATTR-wt positive, an obvious extraneous cause of death was identified and included burning, drowning, hypothermia, suicide, and traffic accident. On the other hand, heart failure due to ATTR-wt was confirmed as the cause of death in 3 of the 32 cases. It is suggested that ATTR-wt may be associated with unexpected death among the elderly.